An underlying defect in PML is that the retinoic acid receptor alpha has lowered affinity for retinoic acid. Based on this, treatment with all trans-retinoic acid (ATRA) has proven a sucessful rational treatment for AML M3, apparently promoting normal differentiation or selective apoptosis. The side effects of ATRA include fever, retention of fluids (which can lead to pulmonary edema) and pseudotumor cerebri.
DIC is common in PML secondary to the release of thromboplastic granules.
Patients may be prophylaxed with heparin.
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